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The features of the mode of delivery in patients with congenital deficiency of coagulation factor VII: case reports


Congenital deficiency of clotting factor VII (FVII) — refers to rare hemorrhagic diatheses, for which no unified approaches to the management of pregnancy and childbirth have been developed. The analysis of hemostasiological management of four pregnant women with congenital hypoproconvertinemia is presented.

Cases reports. All patients had no or minimal history of hemorrhagic syndrome. In two cases, the diagnosis was made during the actual pregnancy. Provision of hemostasis during delivery (two — vaginal delivery, two — cesarean section) was carried out with recombinant activated FVII (rFVIIa): in two cases, once at a dose of 15–30 mcg/kg, in one twice with repeated administration after 12 hours, in another one tranexamic acid was used. There were no hemorrhagic or thrombotic complications. The thromboelastography (TEG) during pregnancy did not correspond to the severity of hypoproconvertinemia: at FVII activity < 5%, the TEG parameters indicated hypercoagulation.

Discussion. There is a weak correlation between the level of FVII activity in plasma and the severity of hemorrhagic syndrome. The decision on the mode of delivery in patients with FVII deficiency is made according to obstetric criteria. In most cases, a single administration of rFVIIa is sufficient at a dose of 15–30 mcg/kg at the beginning of labor or before performing a caesarean section.

About the Authors

A. Yu. Bulanov
Moscow City Hospital No. 52
Russian Federation

Andrey Yu Bulanov, Dr. of Sci. (Medicine), Head of the Mobile resuscitation transfusion team

3, Pekhotnaya str., Moscow, 123182

S. E. Rabotinsky
Moscow City Hospital No. 52
Russian Federation

Stanislav E. Rabotinsky, doctor of the Mobile resuscitation transfusion team

3, Pekhotnaya str., Moscow, 123182

E. L. Bulanova
Moscow City Hospital No. 52; Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation

Ekaterina L. Bulanova, Cand. of Sci. (Medicine), Associate professor, Department of Anesthesiology and Resuscitation; doctor of the Mobile resuscitation transfusion team

3, Pekhotnaya str., Moscow, 123182

8/2, Trubetskaya str., Moscow, 119991

I. B. Simarova
Moscow City Hospital No. 52
Russian Federation

Irina B. Simarova, doctor of the Mobile resuscitation transfusion team

3, Pekhotnaya str., Moscow, 123182

Т. S. Kotomina
Moscow City Hospital No. 52
Russian Federation

Тatiana S. Kotomina, Cand. of Sci. (Medicine), Head of the Maternity Department, branch

3, Pekhotnaya str., Moscow, 123182

I. Yu. Sizova
Moscow City Hospital No. 52
Russian Federation

Irina Yu. Sizova, Cand. of Sci. (Medicine), Head of the Department of Anesthesiology and Resuscitation

3, Pekhotnaya str., Moscow, 123182


1. Artymuk M.V., Astakhov M.V., Belokrinitskaya T.V. et al. Intensive care of disseminated intravascular coagulation syndrome (DIC-syndrome, coagulopathy) in obstetrics. Anesthesiologiya i reanimatologiya. 2019; No. 2: 5-26.

2. Hawke L., Grabell J., Sim W. et al. Obstetric bleeding among women with inherited bleeding disorders: a retrospective study. Haemophilia. 2016; 22(6):906-911. DOI: 10.1111/hae.13067.

3. Kadir R., Chi C., Bolton-Maggs P. Pregnancy and rare bleeding disorders. Haemophilia. 2009;15(5):990-1005. DOI: 10.1111/j.1365-2516.2009.01984.x. Epub 2009 Feb 27.

4. Kulkarni A.A., Lee C.A., Kadir R.A. Pregnancy in women with congenital factor VII deficiency. Haemophilia. 2006;12(4):413-6.

5. Baumann Kreuziger L.M., Colleen T. Morton C.T., Reding M.T. Is prophylaxis required for delivery in women with factor VII deficiency? Haemophilia. 2013; 19(6): 827-832. DOI:10.1111/hae.12167.

6. Sevenet P.O., Kaczor D.A., Depasse F. Factor VII Deficiency: From Basics to Clinical Laboratory Diagnosis and Patient Management. Clin Appl Thromb Hemost. 2017;23(7):703-710. DOI: 10.1177/1076029616670257.

7. Lapecorella M, Mariani G. Factor VII deficiency: defining the clinical picture and optimizing therapeutic options. Haemophilia. 2008 Nov;14(6):1170-5. DOI: 10.1111/j.13652516.2008.01844.

8. Jain Sh., Donkin J., Frey M.-J., Peltier S. et al. Cooper Phenotypical variability in congenital FVII deficiency follows the ISTH-SSC severity classification guidelines: a review with illustrative examples from the clinic Journal of Blood Medicine 2018:9 211–218 DOI: 10.2147/JBM.S157633.

9. Subbaiah M., Kumar S., Roy K.K. et al. Pregnancy outcome in patients with idiopathic thrombocytopenic purpura. Arch Gynecol Obstet. 2014 Feb;289(2):269-73. DOI: 10.1007/s00404-013-2958-x.

10. Hermans C., Hammer F., Lobet S., Lambert C. Subclinical deep venous thrombosis observed in 10% of hemophilic patients undergoing major orthopedic surgery. J. Thromb. Haemost. 2010; 8(5): 1138-1140.

11. Franchini M., Targher G. Montagnana M., Lippi G. Antithrombotic prophylaxis in patients with von Willebrand disease undergoing major surgery: when is it necessary? J. Thromb. Thrombolysis. 2009; 28: 215-219.

12. Ramdass S.K., Loh K.P., Howard L.M. Thrombosis in a bleeding disorder: case of thromboembolism in factor VII deficiency. Clin Case Rep. 2017;5(3):277-279. DOI: 10.1002/ccr3.836

13. Mariani G., Herrman F.H., Schulman S. et al. Thrombosis in inherited factor VII deficiency. J Thromb Haemost 2003; 1:2153-8. DOI: 10.1046/j.1538-7836.2003.00395.x

14. Comes J.F., Devignes J., Thiebaugeorges O. Prophylactic use of a recombinant activated factor VII in delivery haemorrhage by caesarean in a woman with major factor VII deficiency: a case report. Ann Biol Clin. 2011;69(6):713-9. DOI: 10.1684/abc.2011.0623.

15. Zaidi S.M.A., Qureshi R.N., Adil S.N. Factor VII deficiency and pregnancy: A case report and review of literature. J Pak Med Assoc. 2010;60(2):136-8.

16. Tran H.T., Tjønnfjord G.E., Holme P.A. Use of thromboelastography and thrombin generation assay to predict clinical phenotype in patients with severe FVII deficiency. Haemophilia. 2014 Jan;20(1):141-6. DOI: 10.1111/hae.12256.

17. Zozulya N.I., Svirin P.V. Clinical recommendations for the diagnosis and treatment of rare coagulopathies: hereditary deficiency of blood clotting factors II, VII, X.


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